Raymond’s syndrome consists of a collection of clinical findings due to an insult in the mid-pons. One study separates Raymond’s into two distinct types: 1. Classic Raymond’s syndrome, which is characteristically produced via a lesion involving the ipsilateral abducens fascicle and the undecussated corticofacial and corticospinal fibers, and 2. Common Raymond’s syndrome which is similar to the classic type, however, differs in that it spares the corticofacial fibers. Therefore, the clinical manifestations of Classic Raymond’s, which is significantly less common than the common subtype, consists of an ipsilateral abducens palsy (abducens fascicle insult), contralateral central facial paresis (corticofacial insult), and a contralateral hemiparesis (corticospinal insult).
Raymond syndrome was first described in 1895 by Fulgence Raymond. He described a 39-year-old woman with syphilis presenting with an abducens palsy, contralateral facial paresis, and hemiparesis. He suggested that a lesion at the facial decussation in the medial caudal pons could result in these symptoms.
Raymond syndrome is caused by lesions located in the medial ventral pons, which give rise to the characteristic features. There is debate regarding facial involvement, this is due to the uncertain route the corticofacial fibers travel in the brainstem. Lesions involving the corticofacial decussation at the level of the abducens nerve would result in contralateral facial paresis. By contrast, lesions in a more dorsal area would produce an isolated abducens nerve palsy. Raymond syndrome secondary to pontine infarction, hemorrhage from a cavernous hemangioma at the pontomedullary junction, and lacunar infarct has been documented in the literature.
Raymond syndrome is characterized by ipsilateral abducens palsy, contralateral hemiparesis, and facial paralysis. However, pure Raymond syndrome is extremely rare as there are many nuclei and fibers near the root of the abducens nerve. There is a debate regarding facial involvement, due to the uncertain route of the corticofacial fibers in the brainstem. The fibers have multiple routes, including an aberrant bundle near the medial meniscus in the pontine tegmentum. Corticofacial fibers that travel dorsally would be unaffected by a lesion in the ventral pons, resulting in a raymonds syndrome with facial sparing. In patients presenting with a sixth nerve palsy and contralateral hemiplegia, regardless of a central facial palsy, a lesion in the ventral pons should be suspected and raymond syndrome should be considered.
Further classification of Raymond syndrome into two subtypes, the classic type, and the common type, has been proposed. The classic type is characterized by a lesion in the mid pons involving the abducens fascicle and the non-decussated corticofacial and corticospinal fibers. By contrast the common type also involves a lesion affecting the ipsilateral abducens fascicle and non-decussated corticospinal fibers but spares the corticofacial fibers.
There are not clearly defined diagnostic criteria for Raymond syndrome, so the diagnosis is made based on a combination of radiographic and clinical findings. Sixth nerve palsy and contralateral hemiparesis are necessary features. Neuroimaging may demonstrate cytotoxic edema in the ventromedial mid-pons
The treatment depends on the etiology. Most commonly Raymond’s syndrome, like other insults to the brainstem, are a result of vascular disease. Thus, the use of tPA (tissue-type plasminogen activator) can be utilized only if it is administered within 4-5 hours of symptom onset and if there are no other contraindications to therapy (recent hemorrhage, on coagulation therapy, etc.). Because ipsilateral abducens palsy is a signature symptom of Raymond’s syndrome, use of prisms and surgical correction can be helpful to treat bothersome diplopia, especially if it has not resolved months after the incident. Prognosis for recovery is dependent on multiple factors, including baseline function, age of onset, and the ability of the patient to undergo physical therapy.
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